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🌱 來自: systemic sclerosis and scleroderma disorders

Treatment of systemic sclerosis and scleroderma disorders

• Minimize steroid exposure to reduce risk of renal crisis
• Interstitial lung disease:
  • tocilizumab (Lancet Respir Med 2020;8:963), MMF (↓ toxicity vs. cyclophosphamide; Lancet Respir Med 2020;8:304);
  • nintedanib (multikinase inhibitor/antifibrotic) a/w ↓ FVC decline (NEJM 2019; 380:2518).
  • PAH: pulmonary vasodilators (see “Pulm Hypertension”); early Rx a/w better outcomes
• Renal crisis:
  • ACEI (not ARB) for Rx, not prophylaxis (Semin Arthritis Rheum 2015;44:687)
• GI:
  • PPI/H2-blockers for GERD;
  • promotility agents & antibx for bacterial overgrowth
• Cardiac:
  • NSAIDs ± colchicine superior to steroids for pericarditis
• Arthritis:
  • acetaminophen,
  • NSAIDs,
  • hydroxychloroquine,
  • MTX
• Myositis: MTX, AZA, steroids
• Skin:
  • PUVA for morphea. Pruritus: emollients, topical/oral steroids.
  • Fibrosis: MTX; MMF? (Ann Rheum Dis 2017;76:1207; Int J Rheum Dis 2017;20:481).
  • CYC if severe (NEJM 2006;354:2655).
• Auto-HSCT promising for severe disease (NEJM 2018;378:35)